Hautvaskulitiden und systemische Vaskulitiden mit Hautbeteiligung

Zusammenfassung

Vaskulitiden sind entzündliche Erkrankungen der Gefäßwände. Eines der am häufigsten betroffenen Organe ist die Haut. Somit spielt die Haut bei der Differenzialdiagnostik der Vaskulitis eine zentrale Rolle. Der klinische Hautbefund sollte richtungsweisend sein und im Kontext möglicher weiterer Organmanifestationen, der Resultate von Labor- und Bildgebungsdiagnostik sowie immunhistologischer Befunde zur abschließenden Diagnose führen. Die 2012 revidierte International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC 2012) differenziert Vaskulitiden in Klein-, Mittel-, und Großgefäßvaskulitiden. Am häufigsten manifestieren sich an der Haut die Kleingefäßvaskulitiden, welche sich wiederum in Immunkomplex-Vaskulitiden und ANCA-assoziierte Vaskulitiden unterteilen. Da bei den Kleingefäßvaskulitiden die Beteiligung innerer Organe häufig und vielgestaltig ist, sollte immer eine gründliche diagnostische Aufarbeitung erfolgen. Die Polyarteriitis nodosa (PAN) betrifft mittelgroße Arterien und kann isoliert an der Haut als sogenannte kutane PAN auftreten. Großgefäßvaskulitiden wie die Riesenzell-arteriitis gehen eher selten mit kutanen Manifestationen einher.

Summary

Vasculitides are inflammatory diseases of the vessel walls. One of the most frequently affected organs is the skin. Therefore, the skin plays a central role in the differential diagnosis. The pattern of cutaneous manifestations, involvement of other organs, results of laboratory and imaging diagnostics, and finally the immunohistological findings in skin biopsy should lead to the final diagnosis. The 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC 2012) differentiates in small, medium, and large-vessel vasculitides. Skin manifestations are most frequently found in small-vessel vasculitides, which are subdivided into immune complex vasculitides and ANCA-associated vasculitides. Since the involvement of internal organs in small vessel vasculitides is frequent, a rigorous diagnostic work-up should be carried out. Polyarteriitis nodosa (PAN) predominantly affects middle-sized arteries and can occur as an isolated form on the skin as cutaneous PAN. Large vessel vasculitides such as giant cell arteritis are rarely associated with cutaneous manifestations.

Schlüsselwörter

Kutane Vaskulitis - Differenzialdiagnostik - Histologie

Keywords

Cutaneous vasculitis - differential diagnosis - histology

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